Characteristics and risk factors of pulmonary arterial hypertension in patients with primary Sjögren's syndrome

Yan, Shu-Min; Li, Mengtao; Wang, Hui; Yang, Xiaoxi; Wang, Qian; Liu, Yongtai; Lai, Jinzhi; Tian, Zhuang; Song, Hui; Zhao, Yan; Zeng, Xiaofeng

doi:10.1111/1756-185x.13290

Cited by 22 publications

(19 citation statements)

References 35 publications

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“…Regarding extraglandular manifestations of pSS‐PAH, liver involvement is not uncommon. Hepatic injury was detected in 61.5% of patients with pSS‐PAH . This was consistent with the results (53.3%) in our cohort.…”

Section: Discussionsupporting

confidence: 92%

“…Pericardial effusion in CTD‐PAH is associated with significant morbidity and mortality. We identified pericardial effusion as a risk factor for PAH in pSS, which is consistent with preceding studies in SLE‐PAH and pSS‐PAH . The presence of pericardial effusion has also been described in the literature as being associated with poor survival in PAH patients .…”

Section: Discussionsupporting

confidence: 91%

“…In this study, we described the baseline characteristics of this group and identified risk factors for PAH in pSS. Consistent with the results of Launay et al and Yan et al, patients in the pSS‐PAH group were mainly middle‐aged and elderly women, which may be attributed to the gender‐based occurrence of pSS, for which more than 90% of cases are women . Similar to previous studies, the delay between PAH onset and confirmation by RHC was 18.7 ± 19.7 months.…”

Section: Discussionsupporting

confidence: 87%

“…Pulmonary arterial hypertension is a rare but severe complication in patients with pSS. In this study, we described the baseline 13 patients in the pSS-PAH group were mainly middle-aged and elderly women, which may be attributed to the gender-based occurrence of pSS, for which more than 90% of cases are women. 14,15 Similar to previous studies, 12 Pericardial effusion occurs frequently in pSS-PAH patients.…”

Section: Discussionmentioning

confidence: 98%

“…PAH may occur at any time during the course of pSS and was the initial manifestation in 4 of 15 (26.7%) pSS‐PAH patients. Yan et al reported that up to 41.4% (12/29) of pSS‐PAH patients had PAH symptoms as initial manifestations. For patients with only PAH‐related clinical manifestations, pSS‐related screening is also essential.…”

Section: Discussionmentioning

confidence: 99%

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Characteristics and risk factors for pulmonary arterial hypertension associated with primary Sjögren’s syndrome: 15 new cases from a single center

et al. 2019

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Aim This study aimed to retrospectively describe 15 new primary Sjögren's syndrome‐pulmonary arterial hypertension (pSS‐PAH) cases confirmed by right heart catheterization (RHC). Demographic and clinical characteristics were analyzed and risk factors for PAH in pSS were explored. Method We retrospectively described 15 new pSS‐PAH cases confirmed by RHC referred to our institution between January 2013 and March 2018. We present PAH and pSS characteristics, hemodynamic evaluations, medical management, and outcomes. A matched case control study was carried out to determine the risk factors of PAH in pSS compared with pSS‐non‐PAH patients. Results All patients were female with a mean age at PAH diagnosis of 52.9 ± 14.6 years. The delay between the first symptom and PAH diagnosis was 18.7 ± 19.7 months. The most common primary manifestation at PAH onset was exertional dyspnea (13/15). At diagnosis of PAH, PAH was severe with a mean pulmonary artery pressure of 48.8 ± 13.7 mm Hg (range, 27‐72 mm Hg) and a mean cardiac index of 2.3 ± 0.6 L/min/m2 (range, 1.47‐3.41 L/min/m2). Compared with the pSS‐PAH without pericardial effusion, pSS‐PAH with pericardial effusion had larger right arterial (53 [45‐56.75] vs 38 [35.5‐46.5], P = .018) and right ventricular sizes (47 [42.75‐51.25] vs 36 [32.5‐41], P = .007). Compared with the pSS non‐PAH group, we identified 2 risk factors for PAH in pSS: pericardial effusion (odds ratio [OR] [95% CI], 14.29 [1.14‐166.67], P = .039) and liver involvement (OR [95% CI], 14.71 [1.14‐166.67], P = .035). Conclusion For pSS patients, PAH can be the first manifestation. We believe that systemic evaluation, especially in patients with pericardial effusion and liver involvement, is important to identify high‐risk patients for PAH, improving their prognosis.

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Section: Discussionsupporting

confidence: 92%

Section: Discussionsupporting

confidence: 91%